Autoimmune hepatitis-systemic lupus erythematosus (AIH-SLE) overlap syndrome is a rare disease entity. In some cases, a delay in the occurrence of overlap is observed, where the diagnosis of one of the conditions precedes the other. However, other patients have features of both disorders simultaneously upon initial presentation, leading to a direct diagnosis of AIH-SLE overlap syndrome. Thus, the highly variable and non-specific clinical presentations of AIH-SLE overlap syndrome pose a diagnostic challenge that can only be overcome by maintaining a high index of suspicion. The best clinical practice involves the consideration of a wide list of differential diagnoses for each case, tailored toward its unique combination of symptoms and clinical findings. Although challenging, the timely establishment of an accurate diagnosis is crucial as even the etiologies sharing similar autoimmune pathophysiology demonstrate varying responses to the different therapeutic agents. This case report summarizes seven years of a middle-aged female's disease course, starting with her initial presentation, leading to her diagnosis with AIH, moving on to the establishment of overlap with SLE, and monitoring her response to the various treatment regimens she followed over the years. Despite trials of several recommended treatment regimens, the patient did not meet the requirements of remission for either AIH or SLE until the end of this observatory period. For the next steps in her management, several biological agents are under consideration as the existing scientific literature lacks recommendations specific to AIH-SLE overlap syndrome. This case report is intended to enrich the limited scientific literature pertaining to AIH-SLE overlap syndrome by documentation of this patient's individual disease course and review of the existing literature.
Keywords: autoimmune hepatitis; deranged liver function tests; hepatic; immune-mediated disorders; jaundice; joint pain; liver histology; lupoid hepatitis; overlap syndrome; systemic lupus erythematosus.
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